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Choanal atresia, unilateral

MedGen UID:
1843076
•Concept ID:
C5680630
•
Congenital Abnormality
Synonym: choanal atresia, unilateral
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0015312
Orphanet: ORPHA137917

Definition

Unilateral choanal atresia is a, usually sporadic, congenital anomaly that is more commonly seen in females than in males (2:1), where the nose is blocked by bony or soft tissue formed during embryologic development on only one side (more commonly on the right side) and which is characterized by nasal obstruction and rhinorrhea, usually presenting at birth but that may go undetected until a respiratory infection aggravates the condition. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChoanal atresia, unilateral

Professional guidelines

PubMed

Endoscopic treatment of choanal atresia and use of balloon dilation: our experience.
De Vincentiis GC, Panatta ML, De Corso E, Marini G, Bianchi A, Giuliani M, Sitzia E, Tucci FM
Acta Otorhinolaryngol Ital 2020 Feb;40(1):44-49. doi: 10.14639/0392-100X-1567. PMID: 32275648Free PMC Article
International Pediatric Otolaryngology Group (IPOG) consensus recommendations: Diagnosis, pre-operative, operative and post-operative pediatric choanal atresia care.
Moreddu E, Rizzi M, Adil E, Balakrishnan K, Chan K, Cheng A, Daniel SJ, de Alarcon A, Hart C, Hartnick C, Inglis A, Leboulanger N, Pransky S, Rahbar R, Russell J, Rutter M, Sidell D, Smith RJH, Soma M, Spratley J, Thompson D, Trozzi M, Ward R, Wyatt M, Yeung J, Zalzal G, Zur K, Nicollas R
Int J Pediatr Otorhinolaryngol 2019 Aug;123:151-155. Epub 2019 May 13 doi: 10.1016/j.ijporl.2019.05.010. PMID: 31103745
Prognostic Factors and Management of Patients with Choanal Atresia.
Moreddu E, Rossi ME, Nicollas R, Triglia JM
J Pediatr 2019 Jan;204:234-239.e1. Epub 2018 Oct 2 doi: 10.1016/j.jpeds.2018.08.074. PMID: 30291020

Recent clinical studies

Etiology

Prevalence and descriptive epidemiology of choanal atresia and stenosis in Texas, 1999-2018.
Benjamin RH, Marengo LK, Scheuerle AE, Agopian AJ, Mitchell LE
Am J Med Genet A 2024 Jun;194(6):e63549. Epub 2024 Feb 5 doi: 10.1002/ajmg.a.63549. PMID: 38314656Free PMC Article
Pediatric unilateral isolated choanal atresia is not associated with mandibular condyle hypoplasia.
Oh MS, Kadom N, Abramowicz S, Todd NW Jr
Am J Otolaryngol 2021 Jan-Feb;42(1):102824. Epub 2020 Nov 12 doi: 10.1016/j.amjoto.2020.102824. PMID: 33221635
Parapharyngeal Neuroglial Heterotopia: A Case Report and Literature Review.
Alsayid HA, Alnoury IS
Am J Case Rep 2020 Nov 6;21:e926300. doi: 10.12659/AJCR.926300. PMID: 33156818Free PMC Article
CHARGE syndrome.
Blake KD, Prasad C
Orphanet J Rare Dis 2006 Sep 7;1:34. doi: 10.1186/1750-1172-1-34. PMID: 16959034Free PMC Article
Unilateral CHARGE association.
Trip J, van Stuijvenberg M, Dikkers FG, Pijnenburg MW
Eur J Pediatr 2002 Feb;161(2):78-80. doi: 10.1007/s00431-001-0870-z. PMID: 11954755

Diagnosis

Prevalence and descriptive epidemiology of choanal atresia and stenosis in Texas, 1999-2018.
Benjamin RH, Marengo LK, Scheuerle AE, Agopian AJ, Mitchell LE
Am J Med Genet A 2024 Jun;194(6):e63549. Epub 2024 Feb 5 doi: 10.1002/ajmg.a.63549. PMID: 38314656Free PMC Article
Features and Strategies in the Management of Choanal Atresia: A 6-Year Retrospective Analysis.
Kalentakis Z, Stamataki S, Chalkiadakis V, Papapetropoulos N
J Craniofac Surg 2021 Sep 1;32(6):e535-e539. doi: 10.1097/SCS.0000000000007490. PMID: 33770044
Diagnostics and therapy of bilateral choanal atresia in association with CHARGE syndrome.
Koppen T, Bartmann D, Jakob M, Bootz F, Müller A, Dresbach T, Send T
J Neonatal Perinatal Med 2021;14(1):67-74. doi: 10.3233/NPM-200450. PMID: 32741782
Prognostic Factors and Management of Patients with Choanal Atresia.
Moreddu E, Rossi ME, Nicollas R, Triglia JM
J Pediatr 2019 Jan;204:234-239.e1. Epub 2018 Oct 2 doi: 10.1016/j.jpeds.2018.08.074. PMID: 30291020
Choanal atresia.
Nemechek AJ, Amedee RG
J La State Med Soc 1994 Aug;146(8):337-40. PMID: 7930863

Therapy

Prognostic Factors and Management of Patients with Choanal Atresia.
Moreddu E, Rossi ME, Nicollas R, Triglia JM
J Pediatr 2019 Jan;204:234-239.e1. Epub 2018 Oct 2 doi: 10.1016/j.jpeds.2018.08.074. PMID: 30291020
Comprehensive management of congenital choanal atresia.
Brihaye P, Delpierre I, De Villé A, Johansson AB, Biarent D, Mansbach AL
Int J Pediatr Otorhinolaryngol 2017 Jul;98:9-18. Epub 2017 Apr 17 doi: 10.1016/j.ijporl.2017.04.022. PMID: 28583512
Surgery for congenital choanal atresia.
Cedin AC, Atallah AN, Andriolo RB, Cruz OL, Pignatari SN
Cochrane Database Syst Rev 2012 Feb 15;(2):CD008993. doi: 10.1002/14651858.CD008993.pub2. PMID: 22336856
Choanal atresia: embryologic analysis and evolution of treatment, a 30-year experience.
Hengerer AS, Brickman TM, Jeyakumar A
Laryngoscope 2008 May;118(5):862-6. doi: 10.1097/MLG.0b013e3181639b91. PMID: 18197129
CHARGE syndrome.
Blake KD, Prasad C
Orphanet J Rare Dis 2006 Sep 7;1:34. doi: 10.1186/1750-1172-1-34. PMID: 16959034Free PMC Article

Prognosis

Endoscopic treatment of choanal atresia and use of balloon dilation: our experience.
De Vincentiis GC, Panatta ML, De Corso E, Marini G, Bianchi A, Giuliani M, Sitzia E, Tucci FM
Acta Otorhinolaryngol Ital 2020 Feb;40(1):44-49. doi: 10.14639/0392-100X-1567. PMID: 32275648Free PMC Article
Prognostic Factors and Management of Patients with Choanal Atresia.
Moreddu E, Rossi ME, Nicollas R, Triglia JM
J Pediatr 2019 Jan;204:234-239.e1. Epub 2018 Oct 2 doi: 10.1016/j.jpeds.2018.08.074. PMID: 30291020
Choanal atresia: embryologic analysis and evolution of treatment, a 30-year experience.
Hengerer AS, Brickman TM, Jeyakumar A
Laryngoscope 2008 May;118(5):862-6. doi: 10.1097/MLG.0b013e3181639b91. PMID: 18197129
Unilateral CHARGE association.
Trip J, van Stuijvenberg M, Dikkers FG, Pijnenburg MW
Eur J Pediatr 2002 Feb;161(2):78-80. doi: 10.1007/s00431-001-0870-z. PMID: 11954755
Huge nasopharyngeal true teratoma with cleft palate and lip and unilateral choanal atresia.
Choulakis M, Kastanioudakis I, Koudoumnakis E, Ziavra N, Christopoulos G, Skevas A
Otolaryngol Head Neck Surg 2001 May;124(5):584-5. doi: 10.1067/mhn.2001.115369. PMID: 11337670

Clinical prediction guides

Endoscopic Endonasal Repair of Congenital Choanal Atresia: Predictive Factors of Surgical Stability and Healing Outcomes.
Ferlito S, Maniaci A, Dragonetti AG, Cocuzza S, Lechien JR, Calvo-Henríquez C, Maza-Solano J, Locatello LG, Caruso S, Nocera F, Achena A, Mevio N, Mantini G, Ormellese G, Placentino A, La Mantia I
Int J Environ Res Public Health 2022 Jul 26;19(15) doi: 10.3390/ijerph19159084. PMID: 35897454Free PMC Article
Features and Strategies in the Management of Choanal Atresia: A 6-Year Retrospective Analysis.
Kalentakis Z, Stamataki S, Chalkiadakis V, Papapetropoulos N
J Craniofac Surg 2021 Sep 1;32(6):e535-e539. doi: 10.1097/SCS.0000000000007490. PMID: 33770044
Pediatric unilateral isolated choanal atresia is not associated with mandibular condyle hypoplasia.
Oh MS, Kadom N, Abramowicz S, Todd NW Jr
Am J Otolaryngol 2021 Jan-Feb;42(1):102824. Epub 2020 Nov 12 doi: 10.1016/j.amjoto.2020.102824. PMID: 33221635
Nasal dysplasia.
de Blécourt RA, Roddi R, Berg JP, Bloem JJ
Ann Plast Surg 1996 Dec;37(6):633-7. doi: 10.1097/00000637-199612000-00011. PMID: 8988777
A reappraisal of the CHARGE association.
Oley CA, Baraitser M, Grant DB
J Med Genet 1988 Mar;25(3):147-56. doi: 10.1136/jmg.25.3.147. PMID: 3351900Free PMC Article

Recent systematic reviews

Immediate versus delayed surgery in congenital choanal atresia: A systematic review.
Murray S, Luo L, Quimby A, Barrowman N, Vaccani JP, Caulley L
Int J Pediatr Otorhinolaryngol 2019 Apr;119:47-53. Epub 2019 Jan 4 doi: 10.1016/j.ijporl.2019.01.001. PMID: 30665176
Surgery for congenital choanal atresia.
Cedin AC, Atallah AN, Andriolo RB, Cruz OL, Pignatari SN
Cochrane Database Syst Rev 2012 Feb 15;(2):CD008993. doi: 10.1002/14651858.CD008993.pub2. PMID: 22336856

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